Progeria

(Hutchinson-Gilford Syndrome)




cute_girl.jpg
http://www.the-scientist.com/podcasts/theweek/2006/04/27/












Description

Progeria is a rare genetic disease that causes people to age rapidly beginning in early childhood. Usually death is a result of a stroke or Myocardial infarction (heart attack) due to the progressive atherosclerosis, a disease affecting arterial blood vessels.



Symptoms

Symptoms are normal changes you would see in a normally aging person, only that they appear during childhood development. Some of these changes include hair loss, stiff joints, osteoporosis, and atherosclerosis. The connective tissue become tight and early atherosclerosis of blood vessels, leading to abnormal stresses of the heart. Patients with progeria age eight times faster than normal.



Causes

Progeria is not a hereditary disease because most patients die before they are able to reproduce. Progeria is caused by a mutation on the gene LMNA that encodes the protein Lamin A.



Treatment

There is no treatment for Progeria availiable to the public. Scientists are still researching the specific causes of it and seeing if they can correct the issue. However, they are testing the drug farnesyltransferase (FTI) in some young children.



Prognosis

The average patient survives to the early teens, but some can live as long as 30 years old.



Diagnosis

There is no specific test for Progeria. Diagnosis is based on observed symptoms found during a physical.



Statistics

One in 8,000,000 births shows signs of Progeria. Only about 45 people in the world have Progeria today.



creepy_kid.jpg
http://www.sbdevi.org/ProgeriaHGPS.html
http://www.sun-sentinel.com/media/photo/2004-03/11693431.jpg
http://www.sun-sentinel.com/media/photo/2004-03/11693431.jpg



Comments
1. This is very interesting, and we didn't know that it was so rare. Stroke was spelled wrong so I changed it. TF & BS
2. Wow! Those pictures are of the cutest people ever! We didn't know what this disease was until now. It looks great. HK & JM

3.That is so sad that they don't get to really live life very much. We didn't know what this disease was till now, and the pictures were very interesting. Cri-du-Chat (RS and UP)

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Bibliography


American Society for Cell Biology. "Zeroing In On Progeria: How Mutant Lamins Cause Premature Aging." ScienceDaily 14 December 2005. 6 April 2008 < [[|[[|[[|[[|[[|http://www.sciencedaily.com­/<span]]]]]]]]]] style="FONT-FAMILY: 'Comic Sans MS', cursive">releases/2005/12/051214083618.htm >.

"Progeria." All Refer Health. 6 Apr. 2008. 3 Apr. 2008 < http://health.allrefer.com/health/progeria-info.html#definition >.

"Statistics About Progeria." Wrong Diagnosis. 4 Feb. 2008. 4 Apr. 2008 < http://www.wrongdiagnosis.com/p/progeria/stats.htm >.